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Tgf-β1 Inhibits Cftr Biogenesis and Prevents Functional Rescue of ΔF508-Cftr in Primary Differentiated Human Bronchial Epithelial Cells

CFTR is an integral transmembrane glycoprotein and a cAMP-activated Cl(−) channel. Mutations in the CFTR gene lead to Cystic Fibrosis (CF)–an autosomal recessive disease with majority of the morbidity and mortality resulting from airway infection, inflammation, and fibrosis. The most common disease-...

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Main Authors: Snodgrass, Steven M., Cihil, Kristine M., Cornuet, Pamela K., Myerburg, Michael M., Swiatecka-Urban, Agnieszka
Formato: Artigo
Idioma:Inglês
Publicado: Public Library of Science 2013
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Acceso en liña:https://ncbi.nlm.nih.gov/pmc/articles/PMC3650079/
https://ncbi.nlm.nih.gov/pubmed/23671668
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0063167
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